دورية أكاديمية
أعرض في EDS

Cromolyn sodium delays disease onset and is neuroprotective in the SOD1 G93A Mouse Model of amyotrophic lateral sclerosis.

التفاصيل البيبلوغرافية
العنوان: Cromolyn sodium delays disease onset and is neuroprotective in the SOD1 G93A Mouse Model of amyotrophic lateral sclerosis.
المؤلفون: Granucci EJ; Healey Center for ALS at MassGeneral Hospital, Massachusetts General Hospital, Boston, MA, USA., Griciuc A; Genetics and Aging Research Unit, McCance Center for Brain Health, MassGeneral Institute for Neurodegenerative disease, Department of Neurology, Massachusetts General Hospital and Harvard Medical School, Charlestown, MA, 02129, USA., Mueller KA; Healey Center for ALS at MassGeneral Hospital, Massachusetts General Hospital, Boston, MA, USA., Mills AN; Healey Center for ALS at MassGeneral Hospital, Massachusetts General Hospital, Boston, MA, USA., Le H; Genetics and Aging Research Unit, McCance Center for Brain Health, MassGeneral Institute for Neurodegenerative disease, Department of Neurology, Massachusetts General Hospital and Harvard Medical School, Charlestown, MA, 02129, USA., Dios AM; Healey Center for ALS at MassGeneral Hospital, Massachusetts General Hospital, Boston, MA, USA., McGinty D; Genetics and Aging Research Unit, McCance Center for Brain Health, MassGeneral Institute for Neurodegenerative disease, Department of Neurology, Massachusetts General Hospital and Harvard Medical School, Charlestown, MA, 02129, USA., Pereira J; Healey Center for ALS at MassGeneral Hospital, Massachusetts General Hospital, Boston, MA, USA., Elmaleh D; AZTherapies, Boston, MA, USA., Berry JD; Healey Center for ALS at MassGeneral Hospital, Massachusetts General Hospital, Boston, MA, USA., Paganoni S; Healey Center for ALS at MassGeneral Hospital, Massachusetts General Hospital, Boston, MA, USA.; Spaulding Rehabilitation Hospital, Department of PM&R, Harvard Medical School, Boston, MA, USA., Cudkowicz ME; Healey Center for ALS at MassGeneral Hospital, Massachusetts General Hospital, Boston, MA, USA., Tanzi RE; Genetics and Aging Research Unit, McCance Center for Brain Health, MassGeneral Institute for Neurodegenerative disease, Department of Neurology, Massachusetts General Hospital and Harvard Medical School, Charlestown, MA, 02129, USA., Sadri-Vakili G; Healey Center for ALS at MassGeneral Hospital, Massachusetts General Hospital, Boston, MA, USA. gsadrivakili@mgh.harvard.edu.
المصدر: Scientific reports [Sci Rep] 2019 Nov 27; Vol. 9 (1), pp. 17728. Date of Electronic Publication: 2019 Nov 27.
نوع المنشور: Journal Article; Research Support, Non-U.S. Gov't
اللغة: English
بيانات الدورية: Publisher: Nature Publishing Group Country of Publication: England NLM ID: 101563288 Publication Model: Electronic Cited Medium: Internet ISSN: 2045-2322 (Electronic) Linking ISSN: 20452322 NLM ISO Abbreviation: Sci Rep Subsets: MEDLINE
أسماء مطبوعة: Original Publication: London : Nature Publishing Group, copyright 2011-
مواضيع طبية MeSH: Amyotrophic Lateral Sclerosis/*drug therapy , Anti-Inflammatory Agents/*therapeutic use , Cromolyn Sodium/*therapeutic use , Neuroprotective Agents/*therapeutic use, Amyotrophic Lateral Sclerosis/genetics ; Animals ; Anti-Inflammatory Agents/pharmacology ; Cromolyn Sodium/pharmacology ; Cytokines/blood ; Cytokines/genetics ; Cytokines/metabolism ; Female ; Male ; Mice ; Mice, Inbred C57BL ; Motor Neurons/drug effects ; Neuromuscular Junction/drug effects ; Neuroprotective Agents/pharmacology ; Spinal Cord/drug effects ; Spinal Cord/metabolism ; Superoxide Dismutase-1/genetics
مستخلص: Accumulating evidence suggests that neuroinflammatory processes are implicated in the initiation and progression of amyotrophic lateral sclerosis (ALS). Previous reports have demonstrated an increase in microgliosis and astrogliosis in the lumbar spinal cord of SOD1 G93A transgenic mice before the onset of symptoms, a neuroinflammatory response which correlated with disease progression. Importantly, early stage homeostatic microglia enhanced motor neuron survival, while pro-inflammatory microglia were toxic to motor neurons in the SOD1 G93A mice. Recent studies from our group have demonstrated that cromolyn sodium, an FDA approved compound, exerts neuroprotective effects in mouse models of Alzheimer's disease by altering microglial cell activation. Here, we tested the neuroprotective and anti-inflammatory effects of cromolyn sodium in the SOD1 G93A mouse model of ALS. Our results indicate that cromolyn sodium treatment significantly delayed the onset of neurological symptoms, and improved deficits in PaGE performance in both male and female mice, however, there was only an effect on survival in female mice. Furthermore, there was a significant increase in motor neuron survival in the lumbar spinal cord as well as a significant decrease in the denervation of the neuromuscular junction of the tibialis anterior muscle in cromolyn treated transgenic SOD1 G93A mice. Lastly, cromolyn treatment decreased the expression of pro-inflammatory cytokines/chemokines in the lumbar spinal cord and plasma and decreased mast cell degranulation in the tibialis anterior muscle of transgenic SOD1 G93A mice. Together, these findings suggest that cromolyn sodium provides neuroprotection in the SOD1 G93A mice by decreasing the inflammatory response.
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المشرفين على المادة: 0 (Anti-Inflammatory Agents)
0 (Cytokines)
0 (Neuroprotective Agents)
EC 1.15.1.1 (Sod1 protein, mouse)
EC 1.15.1.1 (Superoxide Dismutase-1)
Q2WXR1I0PK (Cromolyn Sodium)
تواريخ الأحداث: Date Created: 20191129 Date Completed: 20201103 Latest Revision: 20210110
رمز التحديث: 20240628
مُعرف محوري في PubMed: PMC6881366
DOI: 10.1038/s41598-019-53982-w
PMID: 31776380
قاعدة البيانات: MEDLINE
الوصف
تدمد:2045-2322
DOI:10.1038/s41598-019-53982-w